Efficacy Of Rituximab For Anti-Neutrophil Cytoplasmic Antibody-Associated Hypertrophic Pachymeningitis: A Case Series

Objective. This study researched the efficacy of rituximab (RIX) in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated hypertrophic pachymeningitis (HP).Methods. Eight patients were identified by retrospective chart review from local registries at four hospitals in Japan. ALL patients met the Chapel Hill 2012 Consensus Conference definitions of ANCA-associated vasculitis and had disease complicated with HP. We assessed the dose of glucocorticoids, C-reactive protein (CRP) levels, Birmingham vasculitis activity score (BVAS) and contrast-enhanced magnetic resonance imaging (MRI) findings of HP before and after RTX administration.Results. Three of eight patients were female. The median age was 68 years. No patients had HP at onset of vasculitis. Two patients had a relapse of HP before RIX administration. RIX was used as the initial treatment for HP in three patient. The daily dose of glucocorticoids, CRP levels and BVAS decreased from baseline to 6 months after RIX treatment in all patients. Evaluation of HP by contrast-enhanced MRI. showed improvement in seven of eight cases. All of seven patients achieved sustained remission at 6 months after RTX treatment. NO serious adverse events were observed in any patient.Conclusion. Our case series highlights the efficacy of RIX in patients with difficult-to-treat ANCA-associated Future prospective studies are warranted to establish B-cell depletion therapy by RIX as a treatment option for ANCA -associated HP.